Acute liver failure in children: The first 348 patients in the pediatric acute liver failure study group

Robert H. Squires, Benjamin L. Shneider, John Bucuvalas, Estella Alonso, Ronald J. Sokol, Michael R. Narkewicz, Anil Dhawan, Philip Rosenthal, Norberto Rodriguez-Baez, Karen F. Murray, Simon Horslen, Martin G. Martin, M. James Lopez, Humberto Soriano, Brendan M. McGuire, Maureen M. Jonas, Nada Yazigi, Ross W. Shepherd, Kathleen Schwarz, Steven LobrittoDaniel W. Thomas, Joel E. Lavine, Saul Karpen, Vicky Ng, Deirdre Kelly, Nancy Simonds, Linda S. Hynan

Research output: Contribution to journalArticle

438 Scopus citations

Abstract

Objectives: To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors. Study design: A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained ≥20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ. Results: The cause of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-acetaminophen drug-related hepatotoxicity (5%), infections (6%), other diagnosed conditions (10%); 49% were indeterminate. Outcome varied between patient sub-groups; 20% with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy. Conclusions: Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.

Original languageEnglish (US)
Pages (from-to)652-658.e2
JournalJournal of Pediatrics
Volume148
Issue number5
DOIs
StatePublished - May 2006

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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    Squires, R. H., Shneider, B. L., Bucuvalas, J., Alonso, E., Sokol, R. J., Narkewicz, M. R., Dhawan, A., Rosenthal, P., Rodriguez-Baez, N., Murray, K. F., Horslen, S., Martin, M. G., Lopez, M. J., Soriano, H., McGuire, B. M., Jonas, M. M., Yazigi, N., Shepherd, R. W., Schwarz, K., ... Hynan, L. S. (2006). Acute liver failure in children: The first 348 patients in the pediatric acute liver failure study group. Journal of Pediatrics, 148(5), 652-658.e2. https://doi.org/10.1016/j.jpeds.2005.12.051